Abstract
We report a patient with continuously recurring hemolytic-uremic syndrome due to factor H deficiency. First at the age of 3 months he showed signs of hemolytic anemia, thrombocytopenia and renal insufficiency, often recurring concomitantly with respiratory tract infections, despite weekly to twice weekly plasma substitution (20 ml/kg body weight). Now at the age of 3.5 years glomerular filtration rate is approximately 50 ml/min/1.73 m2 and psychomotoric development is normal. Since factor H is mainly synthesized in the liver, hepatic transplantation has been proposed as curative treatment. Before justification of liver transplantation as the ultimate treatment for these patients, an international registry should be developed to optimize and standardize therapeutic alternatives.
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Acknowledgements
Information contained in this paper was presented in part and published in abstract form at the annual meeting of the Arbeitsgemeinschaft Pädiatrische Nephrologie (APN), Essen, Germany, 7–9 March 2002 (Nieren- und Hochdruckkrankheiten 2002; 31: 68–69). We thank the nurses and physiotherapist (Ulrike Eisele) of the University Children's Hospital Freiburg for the excellent clinical support.
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Gerber, A., Kirchhoff-Moradpour, A.H., Obieglo, S. et al. Successful (?) therapy of hemolytic-uremic syndrome with factor H abnormality. Pediatr Nephrol 18, 952–955 (2003). https://doi.org/10.1007/s00467-003-1192-3
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DOI: https://doi.org/10.1007/s00467-003-1192-3